Transfusion support in patients with sickle cell disease

Blood transfusions are an integral component of the management of acute and chronic complications of sickle cell disease. Red cells can be administered as a simple transfusion, part of a modified exchange procedure involving manual removal of autologous red cells and infusion of donor red cells, and part of an automated red cell exchange procedure using apheresis techniques. Individuals with sickle cell disease are at risk of multiple complications of blood transfusions, including transfusional hemosiderosis, auto- and alloimmunization to minor red cell and human leukocyte antigens, delayed hemolytic transfusion reactions, and hyper-hemolysis. In low- and middle-income countries in sub-Saharan Africa, where a directed donor system is prevalent and limited laboratory methods are in place to perform extended red cell phenotyping, leukodepletion of cellular products, and infectious disease screening, there are additional challenges to providing safe and adequate transfusion support for this patient population. We review current indications for acute and chronic transfusions in sickle cell disease that are derived primarily from randomized controlled trials and observational studies in children living in high-income countries. We will highlight populations with unique transfusion needs, such as pregnant women and children, as well as the role of the transfusion medicine consultative service for individuals with sickle cell disease planning to have curative hematopoietic stem cell transplantation or gene therapy. Finally, we will discuss risk factors for alloimmunization in individuals with sickle cell disease, emerging new strategies to prevent alloimmunization in this population, and critical gaps in the implementation of transfusion guidelines for sickle cell disease in high- and low-income countries.